Clinical Background

Glucagonomas are pancreatic neuroendocrine tumors (PNETs) that produce excessive amounts of glucagon and are associated with a distinctive clinical syndrome. These tumors have a very high malignant potential, and are the third most common functional PNET.

Epidemiology

Incidence – <1/1,000,000

Age – 50s-60s (median)

Sex – M:F, equal

Risk Factors

Genetic – rarely associated with genetic variations; however, patients diagnosed with MEN1 or von Hippel-Lindau syndrome are at higher risk for glucagonomas

Pathophysiology

Usually sporadic

Tumor of the alpha cells of the pancreatic islets – small number in proximal duodenum

Most frequently malignant, calcified, and located in the body and tail of the pancreas with regional node involvement